Bickerstaff brainstem encephalitis

Bickerstaff's brainstem encephalitis (BBE) is a rare immune disorder in children, characterized by progressive ophthalmoplegia, ataxia, and disturbance of consciousness. Although the etiology of BBE is unknown, it has been linked to various antecedent infections. BBE is associated with the anti-GQ1b IgG antibody, which is present in 66% of a population of patients who had BBE. An autoimmune mechanism produced by microbial infection may trigger its pathogenesis. Intravenous immunoglobulin (IVIg) is effective in immune-mediated disorders such as Fisher syndrome (FS) and Guillain-Barre syndrome (GBS). IVIg is also effective in treatment of BBE, which is similar to the clinical and immunologic features of FS or GBS. Combined therapy of IVIg and high-dose methylprednisolone is a more efficacious therapy than IVIg alone. Most patients make a clinical recovery over six months.